sign in sign up
<<
Home , Neutropenia, Thrombocytopenia

Galicia Clínica | Sociedade Galega de Medicina Interna group of143patientswithautoimmuneneutropenia et al.describe8.4%ofautoimmunethrombocytopenia in a In 1949 Evans and Duane was normal; oropharynx showed erythema butnoexudates; showederythema was normal;oropharynx upper lip (Figure 1); gingival mucosa was friable; otoscopy them with dark appearance) and labial herpes scar on the two ulcerationsontheinnersurface ofthelowerlip(one petechiae through thelimbs andtrunk; oralcavity showed and mucosalmembraneswerehydrated, pink, withsmall rate 90bpm), distress;skin showingnosignsofrespiratory hemodynamically stable(bloodpressure139/89mmHg, heart examination hewasafebrile(37.1ºCauriculartemperature), rash and inner lip mucosal lesions (Figure 1). At physical because ofrecurrentfeverandappearanceacutaneous hewenttotheemergencydepartment medical observation bial lesionsresemblingherpessimplex. Sixdaysafterthefirst benzathine andazithromycin, butrightafterhedevelopedla- improved andfeverresolvedaftertreatmentwithpenicillinG temperature). (38.5-39.0ºCaxillary Symptomsinitially fections, presentedtohisfamilydoctorwithodynophagiaand in - adenoidectomy in 2003 due to recurrent upper respiratory A 20 year-old male, of amidgalectomy and with a past history CASE PRESENTATION ing mostlyabenignconditionwithself-limitedcourse cytopenias aremorecommonininfantsandchildren, be- orsecondary. cellcytopeniascanbeprimary Primary BACKGROUND 2 1 Clara Gomes in ayoungadult: acase-report Severe neutropeniaandthrombocytopenia severe thrombocytopeniaandneutropeniawereknown. airway that was treated with before tory We presentthecaseofayoung adultwithanupperrespira- drugs orhematologicalmalignancies. other conditions, suchasautoimmunediseases, , cytopenias are more common and associatedondary with ages, with beingnotsooftenreported areknowntobethemostaffectedcellline- and erythrocytes acquired hemolyticanemiaandothercytopenias. Recibido: 23/04/2019; Aceptado: 29/04/2109// http://doi.org/10.22546/58/1961 Severe neutropenia andthrombocytopenia inayoungadult: acase-report. Galicia Clin2020;81(4): 115-117 Cómo citaresteartículo: Gomes C, SoaresN, BergantimR, Vilas Boas A. Correspondencia: [email protected] Keywords: Neutropenia, , autoimmunecytopenia neutropeniaandthrombocytopenia.rare associationofsevereprimary The lackofserologicalmarkermakesthisdiagnosisdelayedandresource-consuming. to be of autoimmune etiology. The prompt and sustained response of both cell lines to , reinforced this diagnosis. This case illustrates a very We presentthecaseofa20-year-old malehospitalizedwithsevereneutropeniaandthrombocytopenia. After exclusionofotheretiologies, itwasconcluded ABSTRACT HaematologyDepartment, CentroHospitalarUniversitáriodeSãoJoão, Porto, Portugal InternalMedicineDepartment, CentroHospitalarUniversitáriodeSãoJoão, Porto, Portugal 1 , NeuzaSoares 3 first reported the association of 1 , RuiBergantim

2 ,Boas Abílio-Vilas 4,5 6 . Sec- . Bux 1,2 . decreased numberofleukocytes (showingreactivemono- orcyanocobalamindeficit. Abloodsmearconfirmed kidney functiontests, electrolytesorcoagulationtests;no 33.7 mg/L;nosignificantchange wasfoundonliverpanel, Blood testsshowedsevereneutropenia(0.020x10 or organomegaly;noperipheraledema. abdomen wastenderandpainless, withnopalpablemasses enopathy waspalpable;heartandlungssoundswerenormal; a right non-tender, soft, ad- about 3 cmlong axis axillary trophils/uL) and thrombocytopenia (<10x10 mentation ratewas67mm/1 sedi- hemoglobin levelwasnormal(13.6g/dL);erythrocyte 1

Figure 1. Mucosalulcerationandfriablegingivalmucosa. st h andC-reactiveproteinwas Galicia Clin 2020;81(4):115-117 CASO CLÍNICO 9 platelets/uL); | 9 115 neu- |

PULSE PARA VOLVER AL ÍNDICE CASO CLÍNICO Galicia Clin 2020;81(4):115-117 | 116 creased, asshowninFigures2and3. After thefirstweek Neutropenia andthrombocytopeniapersistedseverelyde- andsmallpetechiae. was treatedwithflucloxacillin. Hepresentedminorgingival a catheter-associated phlebitisthathedeveloped laterand He hadnofeverorsignofactivebacterialinfection, besides time (29days), bloodcellcountsshowednoimprovement. etiology, becauseoftheinitialpresentation. However, overthe afterapresumedinfectiousordrug-related neous recovery while performingdiagnosticworkup. We expectedsponta- illness were present, only supportive treatment was given As thepatientwasclinicallystableandnosignsofcritical cell population. examination andimmunophenotypingrevealedpolyclonalB lymph nodesfoundnothingbutreactivechangesoncytologic mm diameters. Fine-needleaspirationofonetheenlarged right side, the two most evident with 32x17 mm and 35x14 cm). ultrassoundshowedafewadenopathiesonthe Axillary firmed theslightlyincreasedliverandspleen(13.6x15.1x5.3 greatest diameter) and computerized tomography scan con- ous hepatosplenomegaly (18.5 cm and 14.5 cm, respectively, X-ray wasnormal. Abdominal ultrasoundshowedhomogene- range; nootherrelevantfindingonautoimmunestudy. Chest and anti-thyroglobulin was slightly above the normal clear (ANA)titerwas1/100withspeckledpattern the rest of microbiological study were also negative. Antinu- for Epstein-Barrvirus(EBV)werenegative. Bloodculturesand (HCV)directedantibodiesandheterophileantibodytest deficiency virus(HIV), hepatitisBvirus(HBV)andC els andproteinelectrophoresiswerenormal. Humanimmuno- increase inimmaturegranulocyticforms. Immunoglobulinlev- notyping showed no monoclonal cell populations, but a slight and eosinophilsweremorphologicallynormal;immunophe- blasts excess or maturation stop; , lymphocytes morphology, myeloid left shift with reactive precursors, no Bone marrowwasnormocellular, withnormalmegakaryocytes athrombocytes); noevidentschizocytesorcirculatingblasts. cytes), decreasedplateletcount(withanisocytosisandmeg- Severe neutropeniaandthrombocytopeniainayoungadult: acase-report. | Figure 2. Neutrophilcountduringhospitalstayandfollowup. *Treatment withG-CSF;• Treatment withcorticosteroids. after tovalues<10x10 platelets seemed to be spontaneously increasing, but fell right and negative. marrow showed normal cellularity and we found no infectious marrow showednormalcellularityandwefoundnoinfectious countsof80x10 explain peripheraldestructionthroughsplenomegaly could causedecreasedhematopoiesis, andEBVcouldalso lous) or bonemarrow suppression (e.g. HIV, hepatitis, EBV) struction. Invasionofbloodmarrow(e.g. fungalortubercu- result in insult or induce peripheral cell de- exposure were the first diagnostic hypothesis. Both could infectionanduseofantibiotics,respiratory infectionanddrug ously reducedbloodcellcounts. Becauseoftheinitialupper We present the case ofa young patient that showed seri DISCUSSION countsof2.58x10 munosuppressant drugs, thepatientshowsnormalabsolute cess thatlastedfor6months. After 8monthswithnoim- Kg/day and, aftertwo weeks, started a slowweaningpro- The patientwasdischargedhomewithprednisolone1mg/ on plateletcounts, and5daysonabsoluteneutrophilcounts. after1day A promptandsustainedincreasewasobserved mg during3daysandprednisolone1mg/Kg/daythereafter). ogy, corticosteroids were started (methylprednisolone 500 andnoevidenceofotheretiol- lack ofspontaneousrecovery disease orinfiltrativeprocess. After29days, consideringthe hematologic reactive marrowandwithnosignsofaprimary revealinga exams wereconsistentwiththefirstobservation performed, aswellabonemarrowtrephinebiopsy. Both lack of recovery, at day 14, a new bone marrow aspirate was only slightlyandfallbacktobaselinelevels(Figure2). With stimulating factor(G-CSF), neutrophilswouldincreasebut ever thought clinically necessary. With -colony in -inducedneutropenia creased riskofinfection, thisrelationbeingbetterstablished andin- with inabilitytodeliverneutrophilstheperiphery mucosal ulcerationssuggestedalowbonemarrowreserve, Figure 3. Plateletcountduringhospital stay andfollowup. 9 /L. Plateletsweretransfused when- 9 /L. Autoimmune studywasrepeated 9 /L anddecreasedbutstable 9 . Inourpatient, bone 7,8 . Also, -

Galicia Clínica | Sociedade Galega de Medicina Interna Galicia Clínica | Sociedade Galega de Medicina Interna neutropenia mia and, althoughrarely, alsowiththrombocytopeniaand antibodies, beingmoreoftenassociatedwithhemolyticane- lin cancauseanimmunereactionthroughhapten-induced systemic orlocalautoimmunedisease. any recurrenceofimportantcytopeniaandothersign is needed. The patientshouldbefollowedinorder tomonitor delayed, costlyandresource-consuming. Furtherinvestigation marker forthiscondition, andthediagnosticprocessmaybe clude anunderlyingdisorder. There isnospecificserological Our diagnosticworkupwasperformedcarefullyinordertoex- association, especiallywiththeseveritypresentedinthiscase. Autoimmune neutropeniaandthrombocytopeniaarearare CONCLUSION treatment with azithromycin responsible agent. There arereportsofagranulocytosisafter erythematosus orrheumatoidarthritis)hypoxia lupus erythematosus (like brucellosisorEBV), inflammation(asoccursinsystemic but duetoincreaseddemandofbloodcells, suchasinfection conditions unrelatedtobonemarrowhematopoieticfunction hematopoiesis,extramedullary whichisknowntooccurin of opsonized in the spleen or by was unlikely. Splenomegalywouldbeexplainedbyincreased course, suggestedthatthepresenceoflymphoidneoplasms M- ormonoclonalpopulation, aswelltheclinical in theperipheralbloodandbonemarrow, theabsence did notperformspleenbiopsy, butthelackoflymphocytosis disruption, infiltrativeprocessormonoclonalpopulation. We nophenotyping showednoevidenceofdysplasia, architectural nancy: bonemarrowandlymphnodecytologyimmu- There wasnoevidenceofunderlyinghematologicalmalig- ies, whichwerenegativeduringfollow-up. non-specific increasein ANAandanti-thyroglobulinantibod- autoimmunity werenegative, withtheexceptionofslightand systemic autoimmune disease and serological markers of Our patientshowednosymptomssuggestiveoflocalizedor after discontinuationofthisdrugs. combined cytopenia. immune waskeyforthefinaldiagnosisofprimary improvement ofbloodcellcountsafterimmunosuppressive corticosteroids forrecovery, andtheimmediatesustained crucial tosupporttheautoimmunehypothesisandneedof Waiting ofbloodcellcountswas forspontaneousrecovery 11,12 . However, wewouldexpectsomerecovery 10 , and it is known that penicil- 13 . 10. 9. 8. 7. 6. 5. 4. 3. 2. 1. REFERENCES 11. 12. 13.

mucositis. J Antimicrob Chemother2009;63: i36-i40. Blijlevens NMA, LoganRM, NeteaMG.Mucositis: fromfebrileneutropeniato Pathol 2013;44:1154. Weinzieri EP, Arber DA. Bonemarrowevaluationinnew-onsetpancytopenia. Hum 13:10. careteachinghospital.reported todatefromasingletertiary BMCHematol2013; Jain A, NaniwadekarM. An etiologicalreappraisalofpancytopenia–largestseries studiesin143patients.clinical andlaboratory Ann Hematol1991;63: 249-52. Bux J, KisselK, NowakK, Spengel V, Mueller-Eckhardt C. : Hematol Oncol. 1997Sep-Oct;19(5):433-7. Mathew P, ChenG, Wang W. Evanssyndrome: resultsofanationalsurvey. JPediatr Nov;97(5):754-8. Pui CH, Wilimas J, Wang W. Evanssyndromeinchildhood. JPediatr. 1980 1949; 4:1196-213. to activityofthedisease, significanceofthrombocytopeniaandleukopenia. Blood Evans RS, DuaneRT. Acquired hemolyticanemia: antibody relationoferythrocyte adults increaseswithage. Blood. 1999;94: 909-913. Frederiksen H, SchmidtK. The incidenceofidiopathicthrombocytopenicpurpurain neutropenia inchildren: Insightsforclinicians. Ther Adv Hematol6:15-24,2015. Farruggia P, DufourC. autoimmune Diagnosisandmanagementofprimary otitismedia. InterMed2009;48:1089-1091. Kajiguchi T, Ohno T. Azithomycin-related agranulocytosisinanelderlymanwith June;7(6):911-918. pathogenesis, diagnosisandmanagement. J Thromb Haemost. 2009 Aster RH, CurtisBR, McFarlandJG.Drug-induced immunethrombocytopenia: 1981;59: 877-888. Neutropenia afterpenicillins: toxicorimmune-mediated?Klin Wochenschr Neftel KA, Wälti M, SpenglerH, vonFelten A, Weitzman SA, BürgiH, Weck AL. 2010;1: 13-19. Kim CH. hematopoiesis. Homeostatic andpathogenicextramedullary JBloodMed. Galicia Clin 2020;81(4):115-117 Gomes C, etal | 117 |

PULSE PARA VOLVER AL ÍNDICE